My son had a devastating, mysterious illness. The conversation about palliative care knocked me flat

Sudden, unrelenting seizures forced doctors to put 22-year-old Daniel Wong in a medically induced coma. His mother describes the difficulties of dealing with a rare, often-unrecognized disease. Two doctors familiar with the condition, called NORSE, explain how hard it can be to talk with family members about it.

Entering a medical twilight zone

By Nora Wong

On a cloudless September afternoon, I got the call that every parent fears: My son, Daniel, was in a Boston hospital with incapacitating, out-of-the-blue seizures. What unfolded over the next 11 weeks revealed both the best of American medicine as well as the pervasive lack of communication that can harm patients and leave families bewildered.

Three months earlier, Daniel had graduated from Stanford University. He immediately started work at an investment firm for nonprofits in Boston. He was intoxicated with his job, what he called “investing with a heart.” I had just talked with my healthy son the day before he was hospitalized.

My husband and I immediately flew to Boston from our home in New Jersey. We found Daniel in the hospital’s intensive care unit. His doctors were mystified by his seizures, which seemed to have no cause. When ever-higher doses of medications failed to stop the seizures, they placed Daniel in a drug-induced coma to protect his brain.

We summoned our daughter from New York City and our younger son from his college in New Haven. “Daniel is sick,” we told them. “We don’t know what’s wrong. Get here as soon as you can. He may not last the night.”

Three different medical teams cared for Daniel: the intensive care unit team, a neurology team, and an epilepsy team. Like ships passing in the night, each one stopped in to see him at different times. Various specialists helped search for clues to his condition and to battle the complications of treatment. Occasionally, doctors had conflicting opinions, but these differences were never openly discussed.

Instead, the focus was on stopping the seizures. No one talked with us about what Daniel’s future might be once they stopped.

We entered a medical twilight zone. No one knew why Daniel was seizing. And no one seemed sure what to do. Days turned into weeks, and weeks into months.

We waited for test results. We waited for decisions about new treatments. We waited for the outcomes of those treatments. Most of all, we waited for Daniel to wake up. But he didn’t.

Every attempt to lift him from the coma without his seizures returning failed. Once poised to make his mark on the world, Daniel lay silent and still, possessing a strange, immobilized beauty.

Without a diagnosis, we submerged our fears and simply hoped. We tried not to panic. If we let loose our emotions, we knew we would become undone and useless to Daniel. So we measured out each day and focused on the effort to keep him alive.

One day a doctor told us, “We think it’s time to consider comfort care for Daniel.” The doctors had run out of treatments. They could not stop his seizures. And after 78 days in the ICU, Daniel’s body was failing. We were told that if he did survive, he would probably have minimal brain function.

My husband and I were completely caught off guard. The concept of comfort care was the absolute opposite of the fight we had been waging for so long.

Our minds reeled. We couldn’t ask Daniel, whose quirky brilliance defied definition, to analyze the options and tell us what he would be willing to live without. We could only try to imagine his answer. And even at this point, no one had confirmed a diagnosis of what was afflicting Daniel. In this vacuum we were suddenly asked to make a decision that had no retreat.

Parents expect to see their child’s first smile and first steps, to have arguments about driving and dating. The results of a final MRI brain scan left us with something no parent ever expects to do: choose whether to end Daniel’s life, to let him die slowly, or perhaps to have him survive as a wholly different being.

The morning after getting the final MRI results, we stopped aggressive treatment and let the medical team switch to providing palliative care. Daniel died within an hour.

In a daze, we drove to his apartment and packed his belongings. Numb and defeated, we returned home to New Jersey that night.

In the three years since Daniel’s death, I have tried to process what happened. Because one of Daniel’s doctors made a gift in his memory to the American Epilepsy Society, we learned that Daniel died from an epilepsy-related condition. The doctors never actually told us that. They probably assumed we knew, since an epilepsy team led his case. Or each doctor may have assumed another one had already informed us.

We also learned that Daniel probably had a rare disorder called new-onset refractory status epilepticus (NORSE). One of his ICU doctors had mentioned NORSE to me privately at the beginning of Daniel’s hospitalization, but never communicated that to the other medical teams or fully discussed it with us.

I now know that NORSE is a newly defined, poorly understood disorder. Its devastating consequences have long been known, even as effective treatments remain elusive. A registry of NORSE patients has just been launched to learn more about the condition.

Daniel Wong in 2009 after his graduation from high school. (Courtesy Nora Wong)

I realize now how uninformed we were during Daniel’s hospitalization. What happened to him may not have been any different if his doctors had spoken candidly with us along the way. And there’s no doubt that the discussions would have been hugely upsetting to us. But it was painful not to know what was happening and to battle imagined and boundless fears.

I probably added to the miscommunication by asking doctors questions that suggested I had more knowledge than I possessed. I used medical terms for which I had no real understanding. I lacked the basic framework to understand Daniel’s illness and was too overwhelmed and frightened to ask for the full story.

Daniel’s doctors and nurses were devoted to him even though they had never heard his voice or seen his face lit with wonder. Nurses arrived early and left late because of the extra time needed to communicate to the next shift the complications that had arisen each day. Doctors swung by at the end of brutal shifts to check on him before they left for home.

What they didn’t do, though, was fully explain what was happening until the very end. Some may not have understood the progression of the illness themselves. Some may have thought the discussion was someone else’s responsibility. Others may have found it unbearable to utter the words to us until Daniel’s death was imminent.

But we needed those words to help us conceptualize what was happening, even if no one could predict or control what would happen next. We needed time to think through and plan for Daniel’s future when his seizures stopped — and what we would do if they didn’t. Instead, we were left alone to interpret what was happening to our son in the surreal world of a hospital ICU.

After his death, we grieved amid unresolved questions about why Daniel died — including what had caused his seizures.

“I’m not just a math boy anymore. I want the gray,” Daniel once told me after I expressed my dismay that he had dropped out of an advanced math class in high school. He showed me what he meant by focusing his time at Stanford on the inequities in education across the United States.

There are few areas as gray as making end-of-life decisions for your child. I feel Daniel’s hand guiding mine in writing on this topic fraught with moral dilemma, incomplete information, and irrevocable consequences. I sense his stubborn insistence to face the truth no matter how daunting or painful.

NORSE can teach us many lessons because the disorder pushes even the finest hospitals, the most dedicated medical teams, and loving parents beyond their limits. Since Daniel’s death, I have joined with several doctors to form the NORSE Institute. It aims to integrate NORSE research and build a community of doctors, patients, and families.

My family’s story is likely similar to those of others who have had a child, parent, or sibling suffer from a baffling, life-threatening disease. The endings of these stories include not only death but also the end of life as it had been lived before. They expose the reluctance to speak openly about disability and death, which is shared by both doctors and families alike. As hard as it is to face these endings, they will come, even if they are denied or left unspoken.

In NORSE and all cases of life-threatening disorders marked by uncertainty, complete and honest communication among doctors, patients, and families is needed so together they can make the best decisions for the patient.

Nora Wong is the executive director of the NORSE Institute.

Involve the palliative care team early

By Teneille Gofton and Sara E. Hocker

NORSE is a devastating disease for many reasons. It often appears out of the blue, affecting previously healthy young adults. While a few patients wake up and return to their normal lives, most do not. The majority of survivors develop epilepsy. Those who have poorly controlled seizures for weeks or months, if they survive, tend to do so with severe brain injuries. Others die from the complications of treatment or when all options have been exhausted and life-sustaining treatments are withdrawn.

NORSE is an intimidating illness for physicians to encounter and manage. Although there is growing research into NORSE, its causes aren’t fully understood. That means treatment decisions are based on incomplete scientific knowledge.

Medical teams that care for individuals with NORSE often become deeply involved with them and their families. Physicians, nurses, and others interact extensively with the family on a daily basis, sometimes for months. Yet even though we haven’t met our NORSE patients in a conscious state, we feel as though we know them. We see them every day, often more than once. We see the pictures papering their walls, and hear their stories told and retold.

As the weeks go by and repeated attempts to discontinue the medically induced coma without a return of the seizures fail, members of the care team can feel as though they have let down their patients and family members. Some team members may start to question the plan of aggressive treatment, believing that recovery is no longer possible.

The elephant in the room with NORSE is the difficulty in discussing openly with the family that the probability of death and significant brain damage is high. Yet we don’t know in the beginning who will recover fully and who won’t.

The chance of a full recovery is low. Even so, no one wants to give up too soon. We don’t want to lose the family’s trust by alienating them with pessimistic projections. The sad fact is that we really don’t know what the outcome will be until it occurs.

Families don’t always want to know that their loved one might die, or might live with severe brain damage. Some hear it, but the information doesn’t really register. That’s a common response to acute illness, one that lets people maintain hope and composure. With some families, it is impossible to frankly discuss the various outcomes because they are able to focus only on the details of daily care. That coping mechanism helps people avoid looking at the big picture, which can be scary and overwhelming.

One way to bridge the gap between what the health care team is thinking and what the patients and their families understand is to engage a palliative care team to support regular conversations about the treatment plan, possible outcomes, and the goals of care.

All too often, the palliative care team is brought in only when the possibility of a cure has evaporated and death is expected to occur soon. But in situations like NORSE, when disability or death is a likely outcome, palliative care is most beneficial when it is offered early, alongside other medical interventions.

A misconception on the part of patients and families — not to mention health care teams — is that talking about palliative care indicates that doctors have given up all hope and death is imminent. In reality, palliative care can be a vital addition in cases where people survive but undergo a difficult disease course or have ongoing symptoms.

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We believe that palliative care professionals should be part of the care team early in the hospital stay for anyone with NORSE, or any life-altering or life-threatening medical illness because the stress is high in both the family and the health care team. Palliative care providers can support conversations between the health care team and the family, as well as those within the health care team.

For the members of the medical team, such conversations can foster more thorough and open discussions of the case and help build resilience. Among family members, who usually have little or no medical background, palliative care specialists can support and clarify communication with the medical team. They can also help manage the complex physical, cognitive, and emotional challenges when a patient emerges from a coma, or can support both the bereaved family and the distressed health care team if the patient dies.

In NORSE and other life-altering and life-threatening conditions, involving the palliative care team early, alongside state-of-the-art medical care, is likely to benefit patients, their loved ones, and the health care team by bridging the communication gap.

Teneille Gofton, MD, is assistant professor at Western University, Canada, where she specializes in adult neurocritical care and palliative care for patients living with neurological illnesses. Sara E. Hocker, MD, is associate professor of neurology at the Mayo Clinic, where she directs the neurocritical care fellowship. Both are members of the NORSE Institute Medical Advisory Board.

Did any of Daniel’s physicians mention moyamoya? My husband died in 2013 from RSE after having an ischemic stroke four weeks after the first of two encephaloduroarteriosynangiosis (EDAS) surgeries for moyamoya. He’d apparently had TIAs before, but no history of epilepsy.

After two weeks in the ICU and going into seizure every time they tried to lift even ONE of the 4 AEDs he was on, I had to choose between trache-and-PEG or palliative sedation. I chose the latter, as you did, and he died 20 minutes after the ventilator was removed. The only reason I mention moyamoya is it more commonly manifests in children and in Asian populations. It would show up on a CT scan and/or MRI, but most neurologists never see it.

Great article with very thought provoking events. Wondered if it would be of value to add in what words might have worked better in the moments when the staff was trying to impart information to an overwhelmed family. Even if you do refer to palliative care, medical and nursing staff still need practice on how to introduce ongoing information about shifting goals of care and need a mentor to guide them.